Anti-PRPS1 antibody (ab137577)
- Datasheet
- References (1)
- Protocols
Overview
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Product nameAnti-PRPS1 antibody
See all PRPS1 primary antibodies -
DescriptionRabbit polyclonal to PRPS1
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Host speciesRabbit
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Tested applicationsSuitable for: IHC-P, ICC/IF, WBmore details
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Species reactivityReacts with: Human
Predicted to work with: Mouse, Rat, Cow, Xenopus laevis, Xenopus tropicalis
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Immunogen
Recombinant fragment, corresponding to a region within amino acids 142-307 of Human PRPS1.
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Positive control
- 293T, H1299, HeLa, HepG2, Molt 4 and Raji cell lysates; HeLa cells; SW480 xenograft
Properties
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FormLiquid
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Storage instructionsShipped at 4°C. Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.
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Storage bufferpH: 7.00
Preservative: 0.01% Thimerosal (merthiolate)
Constituents: 0.75% Glycine, 1.21% Tris, 10% Glycerol -
Concentration information loading... -
PurityImmunogen affinity purified
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ClonalityPolyclonal
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IsotypeIgG
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Research areas
Associated products
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Compatible Secondaries
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Positive Controls
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Related Products
Applications
Our Abpromise guarantee covers the use of ab137577 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
| Application | Abreviews | Notes |
|---|---|---|
| IHC-P | 1/100 - 1/1000. Perform heat mediated antigen retrieval before commencing with IHC staining protocol using 10mM citrate buffer (pH6.0) or Tris-EDTA buffer (pH8.0). | |
| ICC/IF | 1/100 - 1/1000. | |
| WB | 1/500 - 1/3000. Predicted molecular weight: 34 kDa. |
Target
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FunctionCatalyzes the synthesis of phosphoribosylpyrophosphate (PRPP) that is essential for nucleotide synthesis.
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PathwayMetabolic intermediate biosynthesis; 5-phospho-alpha-D-ribose 1-diphosphate biosynthesis; 5-phospho-alpha-D-ribose 1-diphosphate from D-ribose 5-phosphate (route I): step 1/1.
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Involvement in diseaseDefects in PRPS1 are the cause of phosphoribosylpyrophosphate synthetase superactivity (PRPS1 superactivity) [MIM:300661]; also known as PRPS-related gout. It is a familial disorder characterized by excessive purine production, gout and uric acid urolithiasis.
Defects in PRPS1 are the cause of Charcot-Marie-Tooth disease X-linked recessive type 5 (CMTX5) [MIM:311070]; also known as optic atrophy-polyneuropathy-deafness or Rosenberg-Chutorian syndrome. CMTX5 is a form of Charcot-Marie-Tooth disease, the most common inherited disorder of the peripheral nervous system. Charcot-Marie-Tooth disease is classified in two main groups on the basis of electrophysiologic properties and histopathology: primary peripheral demyelinating neuropathies characterized by severely reduced motor nerve conduction velocities (NCVs) (less than 38m/s) and segmental demyelination and remyelination, and primary peripheral axonal neuropathies characterized by normal or mildly reduced NCVs and chronic axonal degeneration and regeneration on nerve biopsy.
Defects in PRPS1 are the cause of ARTS syndrome (ARTS) [MIM:301835]; also known as fatal ataxia X-linked with deafness and loss of vision. ARTS is a disorder characterized by mental retardation, early-onset hypotonia, ataxia, delayed motor development, hearing impairment, and optic atrophy. Susceptibility to infections, especially of the upper respiratory tract, can result in early death.
Defects in PRPS1 are the cause of deafness X-linked type 1 (DFNX1) [MIM:304500]; also known as congenital sensorineural deafness X-linked 2 (DFN2). It is a form of deafness characterized by progressive, severe-to-profound sensorineural hearing loss in males. Females manifest mild to moderate hearing loss. -
Sequence similaritiesBelongs to the ribose-phosphate pyrophosphokinase family.
- Information by UniProt
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Database links
- Entrez Gene: 781227 Cow
- Entrez Gene: 5631 Human
- Entrez Gene: 19139 Mouse
- Entrez Gene: 29562 Rat
- Entrez Gene: 314140 Rat
- Omim: 311850 Human
- SwissProt: Q2HJ58 Cow
- SwissProt: P60891 Human
see all -
Alternative names
- ARTS antibody
- CMTX5 antibody
- Deafness 2 perceptive congenital antibody
see all
Images
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Western blot - Anti-PRPS1 antibody (ab137577)Anti-PRPS1 antibody (ab137577) at 1/1000 dilution + Molt 4 whole cell lysate at 30 µg
Predicted band size: 34 kDa
10% SDS PAGE -
Immunocytochemistry/ Immunofluorescence - Anti-PRPS1 antibody (ab137577)Immunofluorescent analysis of methanol-fixed HeLa cells labelling PRPS1 with ab137577 at 1/200 dilution. Lower image is costained with Hoechst 33342. -
Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-PRPS1 antibody (ab137577)Immunohistochemical analysis of paraffin-embedded SW480 xenograft labelling PRPS1 with ab137577 at 1/500 dilution.
Protocols
References
This product has been referenced in:
- Ma Y et al. High expression of PRPS1 induces an anti-apoptotic effect in B-ALL cell lines and predicts an adverse prognosis in Chinese children with B-ALL. Oncol Lett 15:4314-4322 (2018). Read more (PubMed: 29541198) »
